Hermansky-Pudlak Syndrome (HPS) is a group of inherited autosomal recessive disorders characterized by mutations affecting the function of lysosome-related organelles (LROs). Patients with HPS often suffer from oculocutaneous albinism, visual impairment, bleeding disorders, and/or inflammatory bowel disease. More importantly, untreatable and progressive pulmonary fibrosis occurs in certain HPS patients, and is the leading cause of death in such patients (Pierson, D. M., et al. Respiration 73, 382-395 (2006)). There are currently no methods to predict which patients will experience pulmonary fibrosis. In addition, little is known about the mechanisms that drive the lung injury and the progressive fibrotic response in HPS patients, and no therapeutics successfully intervene in these responses.